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Laryngeal Synovial Sarcoma: A Rare Clinical Entity

Introduction. Synovial sarcomas (SS) are aggressive malignant soft tissue tumours that are thought to arise from pluripotent mesenchymal cells. Clinical Report. A 20-year-old male presented with an acute onset of respiratory stridor. Computer tomography scanning confirmed a mass arising from the lef...

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Autores principales: Saxby, Clair, Bova, Ron, Edwards, Melanie
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Hindawi Publishing Corporation 2013
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3741611/
https://www.ncbi.nlm.nih.gov/pubmed/23984145
http://dx.doi.org/10.1155/2013/578606
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author Saxby, Clair
Bova, Ron
Edwards, Melanie
author_facet Saxby, Clair
Bova, Ron
Edwards, Melanie
author_sort Saxby, Clair
collection PubMed
description Introduction. Synovial sarcomas (SS) are aggressive malignant soft tissue tumours that are thought to arise from pluripotent mesenchymal cells. Clinical Report. A 20-year-old male presented with an acute onset of respiratory stridor. Computer tomography scanning confirmed a mass arising from the left supraglottic larynx and an emergency tracheostomy was performed. A diagnosis of biphasic synovial sarcoma was formed. A total laryngectomy and left hemithyroidectomy was performed in conjunction with a left modified radical neck dissection. The patient received adjuvant chemotherapy followed by a course of radiotherapy and remains alive and disease free at 18 months after treatment. Discussion. Prognosis for patients with SS is related to primary tumour extent, grade, and size. The presence of the diagnostic translocation, t(X;18), is being targeted and hopefully will lead to the development of new therapeutics (Guadagnolo et al., 2007). Conclusion. Laryngeal SS remains a rare and poorly understood entity. A multidisciplinary approach to treatment is essential and long-term followup is imperative.
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spelling pubmed-37416112013-08-27 Laryngeal Synovial Sarcoma: A Rare Clinical Entity Saxby, Clair Bova, Ron Edwards, Melanie Case Rep Otolaryngol Case Report Introduction. Synovial sarcomas (SS) are aggressive malignant soft tissue tumours that are thought to arise from pluripotent mesenchymal cells. Clinical Report. A 20-year-old male presented with an acute onset of respiratory stridor. Computer tomography scanning confirmed a mass arising from the left supraglottic larynx and an emergency tracheostomy was performed. A diagnosis of biphasic synovial sarcoma was formed. A total laryngectomy and left hemithyroidectomy was performed in conjunction with a left modified radical neck dissection. The patient received adjuvant chemotherapy followed by a course of radiotherapy and remains alive and disease free at 18 months after treatment. Discussion. Prognosis for patients with SS is related to primary tumour extent, grade, and size. The presence of the diagnostic translocation, t(X;18), is being targeted and hopefully will lead to the development of new therapeutics (Guadagnolo et al., 2007). Conclusion. Laryngeal SS remains a rare and poorly understood entity. A multidisciplinary approach to treatment is essential and long-term followup is imperative. Hindawi Publishing Corporation 2013 2013-07-08 /pmc/articles/PMC3741611/ /pubmed/23984145 http://dx.doi.org/10.1155/2013/578606 Text en Copyright © 2013 Clair Saxby et al. https://creativecommons.org/licenses/by/3.0/ This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.
spellingShingle Case Report
Saxby, Clair
Bova, Ron
Edwards, Melanie
Laryngeal Synovial Sarcoma: A Rare Clinical Entity
title Laryngeal Synovial Sarcoma: A Rare Clinical Entity
title_full Laryngeal Synovial Sarcoma: A Rare Clinical Entity
title_fullStr Laryngeal Synovial Sarcoma: A Rare Clinical Entity
title_full_unstemmed Laryngeal Synovial Sarcoma: A Rare Clinical Entity
title_short Laryngeal Synovial Sarcoma: A Rare Clinical Entity
title_sort laryngeal synovial sarcoma: a rare clinical entity
topic Case Report
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3741611/
https://www.ncbi.nlm.nih.gov/pubmed/23984145
http://dx.doi.org/10.1155/2013/578606
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